A case of micturition syncope

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1 A case of micturition syncope Kimberly Bundick, PA-S S L I D E 1

2 Agenda Purpose Utilize case to illustrate classic finding of an interesting pathology Agenda Case study Epidemiology, etiology of disease Diagnosis and treatment Key Takeaways Limit 5 minutes S L I D E 2

3 Chief Complaint Indication for study: 40-year-old male, fainting and tachycardia with urination Additional information: Recent hospitalization for uncontrollable hypertension at OSH 24-hour urine catecholamines were markedly elevated S L I D E 3

4 CT Abdomen Pelvis without Contrast Coronal View S L I D E 4

5 CT Abdomen Pelvis without Contrast Findings and Impression FINDINGS: There is a heterogeneously enhancing mass containing a few calcifications centered within the left anterolateral bladder wall, compatible with a neoplasm. Given the lab abnormalities and history, this likely reflects a bladder pheochromocytoma. This is associated with bilateral pelvic adenopathy, some of which is calcified. Along the left pelvic sidewall, this measures up to 1.7 cm, while along the right pelvic sidewall, this measures up to 1.3 cm. IMPRESSION: Findings highly concerning for a bladder pheochromocytoma with pelvic adenopathy as described S L I D E 5

6 Paragangliomas are rare catecholamine-secreting tumors DESCRIPTION Paragangliomas, much like pheochromocytomas, are rare neuroendocrine, catecholamine-secreting tumors Paragangliomas can be sympathetic (typically symptomatic, located in the sympathetic paravertebral ganglia of thorax, abdomen, and pelvis ), or parasympathetic (typically asymptomatic / nonfunctional and located along the glossopharyngeal and vagal nerves in the neck and at the base of the skull) Distinction from pheos is important given key differences in location, clinical behavior, aggressiveness and metastatic potential, biochemical findings, and association with inherited genetic syndromes EPIDEMIOLOGY Given overlapping characteristics with pheos, specific incidence is unknown but estimated at 0.8 per 100,000 ETIOLOGY ~30 50% are a component of an inherited syndrome; genetic testing is recommended for all patients with a paraganglioma and in selected patients with pheochromocytoma to appropriately characterize risk MALIGNANT POTENTIAL Up to 25% of paragangliomas are malignant; malignant behavior indicated by metastatic spread S L I D E 6

7 Micturition syncope is the classic feature of bladder paragangliomas Production of vasoactive catecholamines leads to symptoms of catecholamine excess including hypertension, episodic headache, sweating, and tachycardia Hypertension is the most common feature Micturition syncope is the clinical hallmark of a catecholaminesecreting bladder paraganglioma The triad of hypertension, hematuria, and symptoms on micturition or sexual activity is considered almost diagnostic of the condition, and is reported variably in 50 to 100 % of patients Some patients have only painless hematuria S L I D E 7

8 Diagnosis is confirmed with lab testing and imaging LAB TESTING Urinary and/or plasma fractionated metanephrines and catecholamines IMAGING Radiologic imaging is an important component of assessment for information Location and a high degree of vascularity may to permit a presumptive preoperative diagnosis of a paraganglioma in setting of catecholamine secreting tumors Appropriate modalities: Ultrasound computed tomography Magnetic resonance imaging (MRI) Angiography Radioisotope imaging using metaiodobenzylguanidine (MIBG) 18F-fluoro-2-deoxyglucose positron emission tomography (FDG-PET) S L I D E 8

9 Treatment of paragangliomas is focused on symptom control and surgical resection if possible There are no curative options for paragangliomas Symptoms of catecholamine excess should be controlled using combined alpha- and beta-adrenergic blockade Primary and metastatic lesions should be resected if possible The clinical course of metastatic paraganglioma is highly variable, with reported five-year survival rates that range widely from 12 to 84% S L I D E 9

10 Key Takeaways Paragangliomas are rare, catecholamine secreting neuroendocrine tumors Present with symptoms of catecholamine excess, with the classic presentation of bladder paragangliomas being micturition syncope Diagnosis is made with a combination of clinical presentation, 24-hour urine catecholamines and imaging Treatment is focused on symptom management and resection, if possible S L I D E 10

11 References Carty, S. et al. Paragangliomas: Epidemiology, clinical presentation, diagnosis, and histology. Uptodate.com, Oct Carty, S. et al. Paragangliomas: Treatment of locoregional disease. Uptodate.com, Oct Carty, S., et al. Paraganglioma and pheochromocytoma: Management of malignant disease. Uptodate.com, Oct S L I D E 11

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